Extramammary Paget's disease natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
If left untreated, the disease always takes a progressive course with no spontaneous recovery. Common complications of extramammary Paget's disease include recurrence of the tumor. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. The prognosis for primary Extramammary Pagets's disease (EMPD) confined to the epidermis is excellent. However, invasive primary extramammary Paget's disease (EMPD) carries a poor prognosis, particularly if lymphovascular invasion is present.[1][2][3][4][5][6]
Natural History
The typical clinical presentation in extramammary Paget's disease consists of multifocal or single lesions that are dry, raised, erythematous, and slow-growing. Over time, the lesions may evolve into crusted, eczematoid, ulcerated, or papillary lesions. Because of their benign appearance, the lesions are often neglected for several years before being presented for medical attention. The duration to local or even metastatic recurrence can vary from months to years, and extramammary Paget's disease can be associated with other cancers in a metachronous or synchronous fashion. With no spontaneous recovery, the disease always takes a progressive course, if not treated early. Because of multifocality, local recurrences are common even when the margins of resection are negative. The average interval between the diagnosis of intraepithelial vulvar extramammary Paget's disease and its sequential progression to invasive carcinoma amounts approximatively to 11 years.[6]
Complications
Recurrence of the disease
Prognosis
- The prognosis for primary Extramammary Pagets's disease (EMPD) confined to the epidermis is excellent. However, invasive primary extramammary Paget's disease (EMPD) carries a poor prognosis, particularly if lymphovascular invasion is present.
- The depth of invasion is an important prognostic factor, with microscopic invasive disease (less than 1 mm dermal invasion) having a more favorable prognosis than lesions showing deeper invasion.
- The prognosis decreases substantially with lymphovascular involvement, with a five-year survival rate of 0% in the presence of inguinal lymph node metastases.
- Lengthy follow up is advocated in all cases of primary extramammary Paget's disease (EMPD) and each patient should be thoroughly investigated to rule out an underlying malignancy, particularly in cases of male genital disease or perianal.
- The prognosis of secondary extramammary Paget's disease (EMPD) depends on the prognosis of the underlying carcinoma but is generally worse than that for primary EMPD.
- The course of extramammary Paget's disease (EMPD) may extend over a period of 10-15 years without evidence of cancer or metastases. In a minority of patients, tumor cells infiltrate the adnexa, dermis, or lymph nodes. Both morbidity and mortality are increased in these patients because of the chemotherapy or extensive surgical treatment that they need.
- The prognosis for extramammary Paget's disease (EMPD) heavily depends on early diagnosis with definitive surgical treatment. Full recovery in extramammary Paget's disease is possible in patients with negative margins after micrographic surgery and purely epidermal. One study showed a mortality of 46% for those with underlying carcinoma and 18% for patients without associated carcinoma.
- Of patients with extramammary Paget's disease, approximately 24% have an associated underlying adnexal adenocarcinoma, which has been associated with a worse prognosis, with mortality rates up to 46%. Up to 12% of patients with extramammary Paget's disease have a concurrent internal malignancy that may be located nearby, and this incidence has been found to be greatest in patients with perianal Paget's disease.
- The prognosis is good when the disease is confined to the epidermis. However, in the presence of dermal invasion, the prognosis is poor.
- Poor prognostic factors for extramammary Paget's disease include the following:[6]
- Primary anorectal extramammary Paget's diesease
- Distant metastasis
- Older age
- Radiotherapy as monotherapy
- Perianal disease
- Dermal invasion
- Lymph node metastasis
- The prognosis remains poor in cases of invasive extramammary Paget's disease
- Extramammary Paget's disease is a rare entity often associated with visceral and adnexal malignancies and a poor prognosis.
- The recurrence rate of extramammary Paget's disease (EMPD) is 30%, even with margin control. The average time to recurrence is 2.5 years, with case reports of more than 10 years follow-up.[1][2][7][4][5]
The relationship between extent of the disease and five-year survival is shown below in a tabular form:
| Extent of disease | Five-year survival |
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References
- ↑ 1.0 1.1 Goldblum JR, Hart WR (1997). "Vulvar Paget's disease: a clinicopathologic and immunohistochemical study of 19 cases". Am J Surg Pathol. 21 (10): 1178–87. PMID 9331290.
- ↑ 2.0 2.1 Goldblum JR, Hart WR (1998). "Perianal Paget's disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma". Am J Surg Pathol. 22 (2): 170–9. PMID 9500217.
- ↑ DeVita, Vincent T., Theodore S. Lawrence, and Steven A. Rosenberg. DeVita, Hellman, and Rosenberg's cancer : principles & practice of oncology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2011. Print.
- ↑ 4.0 4.1 Lloyd J, Flanagan AM (2000). "Mammary and extramammary Paget's disease". J Clin Pathol. 53 (10): 742–9. PMC 1731095. PMID 11064666.
- ↑ 5.0 5.1 Mann, J.; Lavaf, A.; Tejwani, A.; Ross, P.; Ashamalla, H. (2012). "Perianal Paget disease treated definitively with radiotherapy". Current Oncology. 19 (6). doi:10.3747/co.19.1144. ISSN 1198-0052.
- ↑ 6.0 6.1 6.2 Moretto, P.; Nair, V.J.; El Hallani, S.; Malone, S.; Belanger, E.; Morash, C.; Canil, C.M. (2013). "Management of penoscrotal extramammary Paget disease: case series and review of the literature". Current Oncology. 20 (4): 311. doi:10.3747/co.20.1353. ISSN 1718-7729.
- ↑ DeVita, Vincent T., Theodore S. Lawrence, and Steven A. Rosenberg. DeVita, Hellman, and Rosenberg's cancer : principles & practice of oncology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2011. Print.